Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment.

ABSTRACT: Kaposiform lymphangiomatosis (KLA) is a life-threatening rare disease that can cause substantial morbidity, mortality, and social burdens for patients and their families. Diagnosis often occurs long after initial symptoms, and there are few centers in the world with the expertise to diagnose and care for patients with the disease. KLA is a lymphatic anomaly and significant advancements have been made in understanding its pathogenesis and etiology since its first description in 2014. This review provides multidisciplinary, comprehensive, and state-of-the-art information on KLA patient presentation, diagnostic imaging, pathology, organ involvement, genetics, and pathogenesis. Finally, we describe current therapeutic approaches, important areas for research, and challenges faced by patients a

SUBMITTER: McDaniel CG 

PROVIDER: S-EPMC10018800 | biostudies-literature | 2023 Apr

REPOSITORIES: biostudies-literature